Overview
Rheumatic heart disease (RHD) is a a chronic condition caused by irreversible damage to the heart as a result of rheumatic fever.
- Rheumatic fever is an auto-immune mediated condition that results from streptococcal infection, whereby there is production of anti-streptoccocal antibodies, which cross react against cardiac and other tissues
- Previously a widespread disease, which carries a high mortality if untreated, it is now rarely found int he developed world, although it remains common in the developing world and in indigenous populations in Australia.
- The risk of developing rheumatic heart disease after an episode of rheumatic fever is >50%
RHD is the leading cause of heart disease in children and young adults in the developing world.
Rheumatic heart disease is defined as the development of valvular abnormalities following one or more episode of acute rheumatic fever.
- Upon diagnosis of rheumatic heart disease, for many patients, no prior case of rheumatic fever is identified. This suggests a large under diagnosis of episodes of rheumatic fevers.
- Most cases of rheumatic heart disease are detected as the result of a heart murmur
Pathology
Any valve can be affected, but the mitral valve is most common, followed by the aortic, then tricuspid, and finally pulmonary (rare). Any valve can suffer from stenosis, regurgitation, or a combination of both.
Mitral stenosis typically causes more severe disease than mitral regurgitation.
- Suspect rheumatic heart disease in any case of a new murmur, particularly if from an endemic population, or history of rheumatic fever
- Disease progression is variable between geographic location In developed countries, disease progression is often much slower, and rheumatic heart disease is a disease of the over 50s
- In the developing world, it is a disease of children, and it is not uncommon to see cases of severe mitral disease in children as young as 5
- 10 year survival is about 80% if asymptomatic at presentation, but dramatically falls to <15% at 10 years once symptoms develop
Following valvular disease, heart failure typically develops.
Management
- Secondary prophylaxis – with long-term penicillin
- Patients may be considered for valve repair or replacement. In cases where multiple valves are involved, then treatment usually focuses on the most severely affected valve.
- Indications for surgery
- NYHA II – IV, or
- Ejection fraction <60% on echo, or
- Enlarged left ventricle
- New onset AF
- Monitoring of anticoagulation for patients with valve replacements / AF
- Regular cardiology follow-up, with echocardiogram
- If heart failure develops, the treatment is the same as for any other cause of heart failure.
References
- Rheumatic heart disease – UpToDate
- Management of RHD – RHDAustralia
- Murtagh’s General Practice. 6th Ed. (2015) John Murtagh, Jill Rosenblatt
- Oxford Handbook of General Practice. 3rd Ed. (2010) Simon, C., Everitt, H., van Drop, F.